oxford handook of neurology

1. Cutaneous distribution of the nerves of the body First of four views. The anterior cutaneous nerve of the neck has been renamed the transverse cutaneous nerve of the neck. The lower lateral cutaneous nerve of the arm is now recognized as part of the posterior cutaneous nerve of the forearm. Lumboinguinal nerve refers to the femoral branch of the genitofemoral nerve. Reproduced with permission from Haymaker, W. and Woodhall, B. (1998). Peripheral Nerve Injuries: Principles of Diagnosis, 2nd edn. American Association of Neurological Surgeons.

2. Second of four views. See comment opposite regarding the lower lateral cutaneous nerve of the arm. Reproduced with permission from Haymaker, W. and Woodhall, B. (1998). Peripheral Nerve Injuries: Principles of Diagnosis, 2nd edn. American Association of Neurological Surgeons.

3. Cutaneous distribution of the nerves of the body Third of four views. The inferior lateral and inferior medical clunical nerves have been renamed perineal branches of the posterior cutaneous of the thigh. See comment in Fig. A4 legend regarding the lower lateral cutaneous nerve of the arm. Reproduced with permission from Haymaker, W. and Woodhall, B. (1998). Peripheral Nerve Injuries: Principles of Diagnosis, 2nd edn. American Association of Neurological Surgeons.

4. Fourth of four views. The names of some nerves have been changed as follows: The clunical nerves (inferoir lateral and inferior medial) are now termed the perineal branches of the posterior cutaneous nerve of the thigh; the inferior hemorrhoidal nerve is now called the inferior rectal nerve. Reproduced with permission from Haymaker, W. and Woodhall, B. (1998). Peripheral Nerve Injuries: Principles of Diagnosis, 2nd edn. American Association of Neurological Surgeons.

5. OXFORD MEDICAL PUBLICATIONS Oxford Handbook of Neurology

6. Published and forthcoming Oxford Handbooks Oxford Handbook of Clinical Medicine 6/e (also available for PDAs and in a Mini Edition) Oxford Handbook of Clinical Specialties 7/e Oxford Handbook of Acute Medicine 2/e Oxford Handbook of Anaesthesia 2/e Oxford Handbook of Applied Dental Sciences Oxford Handbook of Cardiology Oxford Handbook of Clinical Dentistry 4/e Oxford Handbook of Clinical and Laboratory Investigation 2/e Oxford Handbook of Clinical Diagnosis Oxford Handbook of Clinical Haematology 2/e Oxford Handbook of Clinical Immunology and Allergy 2/e Oxford Handbook of Clinical Surgery 2/e Oxford Handbook of Critical Care 2/e Oxford Handbook of Dental Patient Care 2/e Oxford Handbook of Dialysis 2/e Oxford Handbook of Emergency Medicine Oxford Handbook of Endocrinology and Diabetes Oxford Handbook of ENT and Head and Neck Surgery Oxford Handbook for the Foundation Programme Oxford Handbook of Gastroenterology and Hepatology Oxford Handbook of General Practice 2/e Oxford Handbook of Genitourinary Medicine, HIV and AIDS Oxford Handbook of Geriatric Medicine Oxford Handbook of Medical Sciences Oxford Handbook of Obstetrics and Gynaecology Oxford Handbook of Oncology 2/e Oxford Handbook of Ophthalmology Oxford Handbook of Palliative Care Oxford Handbook of Practical Drug Therapy Oxford Handbook of Psychiatry Oxford Handbook of Public Health Practice 2/e Oxford Handbook of Rehabilitation Medicine Oxford Handbook of Respiratory Medicine Oxford Handbook of Rheumatology 2/e Oxford Handbook of Tropical Medicine 2/e Oxford Handbook of Urology

7. Oxford Handbook of Neurology Hadi Manji Consultant Neurologist and Honorary Senior Lecturer National Hospital for Neurology and Neurosurgery Queen Square, London; and Consultant Neurologist Ipswich Hospital NHS Trust, UK Sen Connolly Consultant in Clinical Neurophysiology St Vincents University Hospital Dublin, Ireland Neil Dorward Consultant Neurosurgeon and Honorary Senior Lecturer Royal Free Hospital London, UK Neil Kitchen Consultant Neurosurgeon, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK Amrish Mehta Consultant Neuroradiologist, Hammersmith Hospitals NHS Trust, London, UK Adrian Wills Consultant Neurologist, Queens Medical Centre, Nottingham, UK

8. iv Great Clarendon Street, Oxford OX2 6DP Oxford University Press is a department of the University of Oxford. It furthers the Universitys objective of excellence in research, scholarship, and education by publishing worldwide in Oxford New York Auckland Cape Town Dar es Salaam Hong Kong Karachi Kuala Lumpur Madrid Melbourne Mexico City Nairobi New Delhi Shanghai Taipei Toronto With offices in Argentina Austria Brazil Chile Czech Republic France Greece Guatemala Hungary Italy Japan Poland Portugal Singapore South Korea Switzerland Thailand Turkey Ukraine Vietnam Oxford is a registered trade mark of Oxford University Press in the UK and in certain other countries Published in the United States by Oxford University Press, Inc., New York Oxford University Press 2007 The moral rights of the authors have been asserted Database right Oxford University Press (maker) First published 2007 All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, without the prior permission in writing of Oxford University Press, or as expressly permitted by law, or under terms agreed with the appropriate reprographics rights organization. Enquiries concerning reproduction outside the scope of the above should be sent to the Rights Department, Oxford University Press, at the address above You must not circulate this book in any other binding or cover and you must impose the same condition on any acquirer British Library Cataloguing in Publication Data Data available Library of Congress Cataloging in Publication Data Data available Typeset by Newgen Imaging Systems (P) Ltd., Chennai, India Printed in Italy on acid-free paper by LegoPrint S.p.A. ISBN 0198509731 (flexicover: alk. paper) 9780198509738 (flexicover: alk. paper) 10 9 8 7 6 5 4 3 2 1

9. 1 v Foreword Pass any young doctor in the corridor of a busy general hospital and the chances are that person will be carrying an Oxford Handbook relevant to their current clinical attachment. Surprise any consultant reviewing notes from a recent clinic in the office and the same book may also be (more discreetly) close at hand. Previously, those dealing with the intri- cacies of clinical neurology were disadvantaged. Now, Hadi Manji, Sen Connolly, Neil Dorward, Neil Kitchen, Amrish Mehta, and Adrian Wills have put right this defect. The team offers expertise in clinical neurology, neurosurgery, neurophysiology, and neuroradiology. And, as consultants working in busy clinical neuroscience centres, each brings to his contribu- tion the discipline of a classical approach to the neurological encounter together with pragmatism, much common sense, and a good deal of clinical experience. This is not a book to read expecting the rich and discursive prose narra- tives of the eloquent clinical expositor; nor, equally, one in which to be ensnared by the weeds of descriptive reflexology or shackled by the competitive impedimenta of eponymous hagiographyalthough a useful appendix lists some names that have echoed through the corridors of neurological establishments down the ages. Rather, it is a book for both the specialist and generalist to consult when faced with the typical, but nonetheless complex, presentations of neurological and neurosurgical disorders; one from which to be reminded of how best to investigate and manage the many conditionscommon and otherwisethat affect the central and peripheral nervous systems and muscle; and one that wisely sets out what to expect from laboratory investigations, and how these inform clinical formulations that remain the substance of clinical neurology. Bullet points, lists, and algorithms for diagnosis and management may not make for bedtime reading but they do provide an economic and invaluable synthesis for others of what needs to be known in order to manage diseases of the nervous system effectively. Having done this successfully for themselves on many occasions in the clinic and on the wards, the team of experts now passes on its experience and under- standing of neurological and neurosurgical disease to a wider readership. Do not look for copies of the Oxford Handbook of Neurology sitting undis- turbed on dusty office shelves. This book will only be found alongside the many dog-eared and well-thumbed copies of its 35 companion volumes in the pockets and on the desktops of busy students of neurological disease. Professor Alastair Compston University of Cambridge October 2006

10. vi Oxford University Press makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check the product information and clinical procedures with the most up-to-date published product information and data sheets provided by the manufacturers and the most recent codes of conduct and safety regulations. The authors and the publishers do not accept responsibility or legal liability for any errors in the text or for the misuse or misapplica- tion of material in this work.

11. 1 vii Preface General physicians have always found neurology difficult and perhaps intimidating. This is a reflection of inadequate training and perhaps per- petuated by the neurologists of a bygone era. Neurology still remains the most clinical of the medical subspecialitiesinvestigative tools such as MRI and DNA analysis will never replace the basic neurological history- taking and examination, which when performed skilfully, is wonderful to watch. This is not some voodoo technique revealed to the chosen few but can be learnt from good role models and practise. Even today, neurological training remains a clinical apprenticeship with hints and clinical handles that are passed down from teacher to pupil and are not in the standard textbooks. In this book we have tried to pepper these in when appropriate. In keeping with the style of the Oxford Handbook series the format is necessarily didactic and hopefully clear for the reader when faced with a patient with neurological symptoms and signs. Neurology and neurologists have had a reputation for being elephan- tine in their diagnostic skills but murine in their therapeutic strategies. This has changed with numerous treatment options now being available. Although neither dramatic in their benefit nor curative, options now exist for patients with multiple sclerosis, Alzheimers disease, motor neuron disease, Parkinsons disease, and ischaemic stroke. Our hope is that this book will go some way to smooth the neurological pathways for juniors in training and perhaps even some senior colleagues! few patients oblige with the symptoms it is their duty to have and not many refrain from complaining of those they ought not to have. When I tried to teach the art of medical diagnosis to students, I often used to ask them this riddle: what runs about farm yards, flaps its wings, lays eggs and barks like a dog? the answer is a hen! Usually one of the more earnest and innocent of the students would say: but sir! I dont understand the bit about barking like a dog. Ah yes, I must explain. That was just put in to make it difficult. [Richard Asher quoted in British Medical Association (1984). A sense of Asher; a new miscellany. BMA, London.] Hadi Manji September 2006

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13. 1 ix Acknowledgements Dr Mike Lunn and Dr Andrew Graham for reading the manuscript and making helpful suggestions; Dr Chris Hawkes for his help with Clinical Pearls; Catherine Barnes and Elizabeth Reeve for their steadfast support and encouragement.

15. 1 xi Contents Symbols and abbreviations xiii Detailed contents xxv 1 Neurological history and examination 2 Neuroanatomy 3 Common clinical presentations 4 Neurological disorders 5 Neurosurgery 6 Clinical neurophysiology 7 Neuroradiology Appendix 1: Neurological disability scales Appendix 2: Clinical pearls Appendix 3: Neurological eponyms Appendix 4: Useful websites Index 525 1 33 55 103 315 425 479 507 511 515 523

17. 1 xiii Symbols and abbreviations Symbols and abbreviations greater than or equal to less than or equal to d decreased i increased AC air conduction ACE angiotensin converting enzyme ACh acetylcholine AChI acetylcholinesterase inhibitor AChR acetylcholine receptor ACom anterior communicating artery ACST Asymptomatic Carotid Surgery Trial AD autosomal dominant ADC apparent diffusion coefficient (map) ADCA autosomal dominant cerebellar ataxia ADEM acute disseminated encephalomyelitis ADL activities of daily living ADM abductor digiti minimi (muscle) A & E accident and emergency (department) AED anti-epileptic drug AF atrial fibrillation AHB abductor hallucis brevis AIC anterior iliac crest AIDP acute inflammatory demyelinating polyneuropathy AIDS acquired immune deficiency syndrome AION anterior ischaemic optic neuropathy ALL anterior longitudinal ligament ALS amyotrophic lateral sclerosis AMAN acute motor axonal neuropathy AMSAN acute motor and sensory axonal neuropathy ANA antinuclear antibody ANCA anti-neutrophil cytoplasmic antibody

18. SYMBOLS AND ABBREVIATIONSxiv AP anteroposterior APB abductor pollicis brevis (muscle) ApoE apolipoprotein E APP amyloid precursor protein AR autosomal recessive ASA anterior spinal artery ASDH acute subdural haematoma ASO ankle-stablizing orthosis ATLS advanced trauma life support (protocol) AV arteriovenous AVF arteriovenous fistula AVM arteriovenous malformation BAER brainstem auditory evoked response BBB bloodbrain barrier BC bone conduction bd twice a day BE bacterial endocarditis BETS benign epiletiform transients of sleep BHCG beta human chorionic gonadotrophin BIH benign intracranial hypertension BMD Becker muscular dystrophy BMI body mass index BP blood pressure BPPV benign paroxysmal positional vertigo BSE bovine spongiform encephalopathy CADASIL cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy CAN chronic axonal neuropathy c-ANCA cytoplasmic anti-neutrophil cytoplasmic antibody CB conduction block CBD corticobasal degeneration CCA common carotid artery CCF carotid cavernous fistula CE contrast-enhanced (MRI) CEA carcinoembryonic antigen CEO chronic external ophthalmoplegia CH cluster headache CIDP chronic inflammatory demyelinating polyneuropathy

19. SYMBOLS AND ABBREVIATIONS1 xv CJD CreutzfeldtJakob disease CK creatine kinase CMAP compound muscle action potential CMT CharcotMarieTooth disease CMV cytomegalovirus CNE concentric needle electrode CNS central nervous system COC combined oral contraceptive COMT catechol-O-methyltransferase COX cyclo-oxygenase CPA cerebellopontine angle CPAP continuous positive airway pressure CPEO chronic progressive external ophthalmoplegia CPK creatine phosphokinase CPP cerebral perfusion pressure CR controlled-release CSF cerebrospinal fluid CT computerized tomography CTA computerized tomography angiography CV conduction velocity CVS cardiovascular system Cx cervical (spine) CXR chest X-ray DA dopamine DAI diffuse axonal injury DaT dopamine transporter dAVF dural arteriovenous fistula DCLB dementia with cortical Lewy bodies ddC 2, 3-dideoxycytidine ddI 2, 3-dideoxyinosine DHE dihydroergotamine DIC disseminated intravascular coagulation DILS diffuse inflammatory lymphocytosis syndrome DIO dorsal interosseous (muscle) DIP distal interphalangeal DLB dementia with Lewy bodies DM diabetes mellitus or dermatomyositis

20. SYMBOLS AND ABBREVIATIONSxvi DMD Duchenne muscular dystrophy DML distal motor latency DMT disease-modifying treatment DNET dysembryoplastic neuroepithelial tumour DRD dopa-responsive dystonia DRPLA dentatorubral pallidoluysian atrophy DSA digital subtraction angiography d4T 2, 3-didehydro-3'-deoxythymidine DVA developmental venous anomaly DVLA Driver and Vehicle Licensing Agency DVT deep vein thrombosis DWI diffusion-weighted image DXT deep X-ray therapy EA episodic ataxia (EA1, EA2) EAM external auditory meatus EBV EpsteinBarr virus ECG electrocardiogram ECT electroconvulsive therapy EDB extensor digitorum brevis (muscle) EDH extradural haematoma EEG electroencephalogram EHL extensor hallucis longus (muscle) EMG electromyography ENA extractable nuclear antigen EOM eye movement channel (in EEG) EP evoked potential EPC epilepsia partialis continua EPP end plate potential ERG electroretinography ESR erythrocyte sedimentation rate ET essential tremor EVD extraventricular drain FBC full blood count FEV1 forced expiratory volume in 1 second FDG fluorine-18 labelled deoxyglucose FDP flexor digitorum profundus FDS flexor digitorum superficialis

21. SYMBOLS AND ABBREVIATIONS1 xvii FH family history FLAIR fluid attenuated inversion recovery (MRI) FLARE fast low-angle recalled echoes (MRI) FM foramen magnum fMRI functional magnetic resonance imaging FP-CIT fluoropropyl-2-carbomethoxy-3-(4-[125I]- iodophenyl)tropane FSH facioscapulohumeral (dystrophy) FTD frontotemporal dementia FVC forced vital capacity GAD glutamic acid decarboxylase GBS GuillainBarr syndrome GCS Glasgow Coma Scale Gd gadolinium GE gradient echo GEN gaze-evoked nystagmus GI gastrointestinal GP general practitioner GPi globus pallidus internus GSS GerstmannStrausslerScheinker (syndrome) GT glutamyl transferase GTN glyceryl trinitrate GTP guanosine triphosphate GTT glucose tolerance test HAART highly active retroviral therapy HAD HIV-associated dementia Hb haemoglobin HD Huntingtons disease HDL high density lipoprotein HDU high-dependency unit HHV6 human herpesvirus 6 HI head injury HIV human immunodeficiency virus HLA human leucocyte antigen (system) HNPP hereditary neuropathy with liability to pressure palsies HMSN hereditary motor and sensory neuropathy HOCM hypertrophic obstructive cardiomyopathy HRT hormone replacement therapy

22. SYMBOLS AND ABBREVIATIONSxviii HSAN hereditary sensory and autonomic neuropathy HSE herpes simplex encephalitis HSN hereditary sensory neuropathy HSV herpes simplex virus 5-HT 5-hydroxytryptamine HTLV-I human T-cell lymphocytotrophic virus type I hyperKPP hyperkalaemic periodic paralysis hypoKPP hypokalaemic periodic paralysis IAC internal auditory canal IBM inclusion body myositis ICA internal carotid artery ICH intracerebral haemorrhage ICP intracranial pressure ICU intensive care unit Ig immunoglobulin (IgA, IgM, etc.) IHD ischaemic heart disease IHS International Headache Society IIH idiopathic intracranial hypertension IM intramuscular INO internuclear ophthalmoplegia INR international normalized ratio IO inferior oblique (muscle) IP interphalangeal (joint) IPD idiopathic Parkinsons disease IPNV isolated peripheral nerve vasculitis IQ intelligence quotient IR inferior rectus (muscle) ISH idiopathic stabbing headache ITU intensive therapy unit IV intravenous IVDU intravenous drug user JME juvenile myoclonic epilepsy KSS KearnsSayre syndrome LA local anaesthetic LDL low density lipoprotein LEMS LambertEaton myasthenic syndrome LFT liver function test

23. SYMBOLS AND ABBREVIATIONS1 xix LGMD limbgirdle muscular dystrophy (LGMD1A, LGMD1B, etc.) LHON Lebers hereditary optic neuropathy LMN lower motor neuron LOC loss of consciousness LP lumbar puncture LR lateral rectus (muscle) LVF left ventricular failure MAG myelin-associated glycoprotein MAOI monoamine oxidase inhibitors MCA middle cerebral artery MCV mean corpuscular volume or motor conduction velocity MD myotonic dystrophy MELAS mitochondrial encephalopathy with lactic acidosis and stroke-like episodes MERRF mitochondrial epilepsy with ragged red fibres MG myasthenia gravis MGUS monoclonal gammopathy of unknown significance MI myocardial infarction or myoinositol min minute/s MIP maximum intensity projection (MRI) MMN multifocal motor neuropathy MMNCB multifocal motor neuropathy with conduction block MMSE mini-mental state examination MND motor neuron disease MNGIE mitochondrial myopathyneuropathyGI dysmotility encephalopathy MP metacarpophalangeal (joint) MPR multiplanar reformation (CT) MPTP 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine MR medial rectus (muscle) or magnetic resonance MRA magnetic resonance angiography MRI magnetic resonance imaging MRS magnetic resonance spectroscopy MRSA methicillin-resistant Staphylococcus aureus MRV magnetic resonance venography MS multiple sclerosis MSA multiple system atrophy MSA-C multiple system atrophy, olivo-ponto-cerebellar variant

24. SYMBOLS AND ABBREVIATIONSxx MSA-P multiple system atrophy, parkinsonian variant MSLT multiple sleep latency test MSU midstream urine MUAC motor-unit action potential MUP motor unit potential MuSK muscle-specific kinase NAA N-acetyl aspartate NAP nerve action potential NARP neuropathyataxiaretinitis pigmentosa NBCA N-butyl-cyanoacrylate (glue) NCS nerve conduction studies NCT non-contrast computerized tomography NEAD non-epileptic attack disorder neuro obs neurological observations NF neurofibromatosis (NF1, NF2) NG nasogastric (tube) NINDS National Institute of Neurological Disorders and Stroke (USA) NIV non-invasive ventilation NMDA N-methyl-D-aspartate NMJ neuromuscular junction NMO neuromyelitis optica NPH normal pressure hydrocephalus NSAID nonsteroidal anti-inflammatory drug NTD neural tube defect O2 sat. oxygen saturation OCB oligoclonal band od once a day ON optic neuritis OP opening pressure OPCA olivopontocerebellar atrophy OSAHS obstructive sleep apnoea/hypopnoea syndrome OT occupational therapist PaCO2 arterial carbon dioxide tension p-ANCA perinuclear anti-neutrophil cytoplasmic antibody PANK pantothenate kinase PaO2 arterial oxygen tension PC phase contrast

25. SYMBOLS AND ABBREVIATIONS1 xxi PCNSL primary CNS lymphoma PCO2 carbon dioxide tension Pcom posterior communicating (artery) PCR polymerase chain reaction PD Parkinsons disease or proton density PE pulmonary embolism or plasma exchange PEG percutaneous endoscopic gastrostomy PET positron emission tomography PICA posterior inferior cerebellar artery PIPJ proximal interphalangeal joint PK protein kinase PLD peripheral labyrinthine disorder PLEDS periodic lateralizing epileptiform discharges PLL posterior longitudinal ligament PM polymyositis PMA progressive muscular atrophy PMH past medical history PML progressive multifocal leucoencephalopathy PNET primitive neuroectodermal tumours PNS peripheral nervous system PO orally, by mouth PO2 oxygen tension POEMS polyneuropathyorganomegalyendocrinopathy monoclonal gammopathyskin changes POP progestogen only pill PPMS primary progressive multiple sclerosis PPRF paramedian pontine reticular formation PR per rectum (via the rectum) PROGRESS Perindopril Protection Against Recurrent Stroke Study PROMM proximal myotonic myopathy PrP prion protein PSA prostate-specific antigen PSP progressive supranuclear palsy PV per vagina (via the vagina) PWI perfusion-weighted imaging (MRI) qds four times a day RAPD relative afferent pupillary defect RAS reticular activating system

26. SYMBOLS AND ABBREVIATIONSxxii RBC red blood cell RBD REM sleep behaviour disorder RCT randomized controlled trial REM rapid eye movement (sleep) RIG radiologically inserted gastrostomy RNS repetitive nerve stimulation RR relative risk or respiratory rate RRMS relapsing/remitting multiple sclerosis RTA road traffic accident rt-PA recombinant tissue plasminogen activator RX treatment SA sinoatrial (node) SAD seasonal affective disorder SAH subarachnoid haemorrhage SALT speech and language therapist SaO2 arterial oxygen saturation SC subcutaneous SCA spinocerebellar ataxia SCLC small cell lung cancer SCM sternocleidomastoid (muscle) SCV sensory conduction velocity SDH subdural haematoma SE spin echo SERMS selective (o)estrogen receptor modulator SFEMG single fibre electromyography SIADH syndrome of inappropriate antidiuretic hormone SjvO2 jugular venous oxygen saturation SLE systemic lupus erythematosus SMA spinal muscular atrophy SN substantia nigra SNAP sensory nerve action potential SO superior oblique (muscle) SOD1 superoxide dismutase 1 SOMI sterno-occipito-mandibular immobilizer (brace) SPECT single photon emission computerized tomography SPMS secondary progressive multiple sclerosis SR superior rectus (muscle) or slow-release

27. SYMBOLS AND ABBREVIATIONS1 xxiii SSEP somatosensory evoked potential SSPE subacute sclerosing panencephalitis SSRI selective serotonin reuptake inhibitor SSPE subacute sclerosing panencephalitis STICH Surgical Trial in Intracerebral Haemorrhage STN subthalamic nucleus SUDEP sudden unexpected death in epilepsy SUNCT short-lasting unilateral neuralgiform headache with conjunctival injection and tearing SWJ square wave jerk SXR skull X-ray T4 thyroxine TB tuberculosis T/C tonicclonic (seizure) TCA tricyclic antidepressant tds three times a day TG trigeminal TIA transient ischaemic attack tid three times a day TLE temporal lobe epilepsy TM tympanic membrane TMJ temporomandibular joint TOE transoesophageal echocardiogram TOF time of flight (in MRI) TPHA Treponema pallidum haemagglutination assay (syphilis) TPMT thiopurine methyltransferase TVO transient visual obscuration T1W T1-weighted (MRI) T2W T2-weighted (MRI) U & E urea and electrolytes UMN upper motor neuron UPDRS unified Parkinsons disease rating scale UPSIT University of Pennsylvania smell identification test URTI upper respiratory tract infection USS ultrasound scan UTI urinary tract infection UV ultraviolet VA visual acuity or ventriculo-atrial

28. SYMBOLS AND ABBREVIATIONSxxiv VC vital capacity vCJD variant CJD VDRL Venereal Disease Research Laboratory (test for syphilis) VEP visual evoked potential VER visual evoked response VHL Von HippelLindau disease VIM ventral intermediate (thalamic nucleus) VLCFA very-long-chain fatty acid VLDL very low density lipoprotein VMA vanillylmandelic acid VP ventricular peritoneal VZV varicella zoster virus WBC white blood cell WCC white cell count WFNS World Federation of Neurological Surgeons XL extended release (drug) Symbols and abbreviations

29. 1 xxv Detailed contents Symbols and abbreviations xiii 1 Neurological history and examination 1 Principles of neurological history taking 2 The general examination 4 Cranial nerve 1 (olfactory nerve) 6 Cranial nerve 2 (optic nerve and visual pathway) 8 Cranial nerves 3 (oculomotor), 4 (trochlear), and 6 (abducens) 12 Cranial nerves 5 and 712 16 Examination of the upper and lower limbs 18 Bedside cognitive testing, including language 24 The mini-mental state examination (MMSE) 30 2 Neuroanatomy 33 The cranial cavity 34 Dermatomes of the upper and lower limbs 36 Innervation of the upper limbs 38 Innervation of the lower limbs 46 Cross-sections of the brain and spinal cord 52 3 Common clinical presentations 55 Loss of consciousness 56 Acute vertigo 58 Acute headache (thunderclap headache) 62 Acute neuromuscular weakness 64

30. DETAILED CONTENTSxxvi Detailed contents Acute focal neurological syndromes 68 Spastic paraparesis 70 Ataxia 72 Acute visual failure 76 Coma 80 Coma prognosis 84 Excessive daytime sleepiness 86 Tremor 90 Tics 94 Chorea and athetosis 96 Myoclonus 98 Dystonia 100 4 Neurological disorders 103 Cerebrovascular disease: stroke 104 Management of stroke 108 Prevention of ischaemic stroke 110 Cerebral venous thrombosis 112 Images in cerebrovascular disease 114 Dementia: introduction 124 Alzheimers disease 126 Frontotemporal dementia 128 Other dementias 130 CreutzfeldtJakob disease (CJD) 132 Epilepsy: introduction 136 Management of epilepsy 138 Women and epilepsy 142 Seizures versus dissociative non-epileptic attack disorder (NEAD) or pseudoseizures 144 Management of status epilepticus 146

31. DETAILED CONTENTS1 xxvii Headache: migraineintroduction and clinical features 148 Migraine: differential diagnosis, investigations, and IHS criteria 150 Management of acute migraine: the stepped care regimen 152 Management of migraine prophylaxis 154 Migraine and women 156 Primary short-lasting headaches 160 Cluster headache 162 Trigeminal neuralgia 164 Idiopathic intracranial hypertension (IIH) 166 Parkinsonism and Parkinsons disease (PD): introduction 170 Clinical features of parkinsonism and PD 172 Differential diagnosis of PD and investigation 174 Drug-induced parkinsonism 178 Medical management of PD 180 Surgical treatment of PD 184 Management of other problems in PD 186 Multiple system atrophy (MSA) 188 Progressive supranuclear palsy (PSP) 190 Corticobasal degeneration (CBD) 192 Peripheral nerve disorders: introduction and clinical approach 194 Diagnosis of peripheral nerve disorders 196 Investigations in peripheral nerve disorders 198 Diabetic neuropathies 200 GuillainBarr syndrome (GBS) 202 Chronic inflammatory demyelinating polyneuropathy (CIDP) 206 Multifocal motor neuropathy with conduction block (MMN-CB) 210

32. DETAILED CONTENTSxxviii Vasculitic neuropathy 212 Muscle disorders: classification and features 214 Muscle disorders: investigations 216 Dermatomyositis, polymyositis, and inclusion body myositis 220 Motor neuron disease: introductions and clinical features 224 Motor neuron disease: investigations and management 226 Multiple sclerosis: introduction and clinical features 228 Multiple sclerosis: investigations and diagnosis 230 Multiple sclerosis: management 236 Myasthenia gravis: introduction, clinical features, and investigations 238 Myasthenia gravis: management 240 Paraneoplastic disorders: introduction 244 Paraneoplastic syndromes: central nervous system 246 Paraneoplastic syndromes: peripheral nervous system 250 Paraneoplastic syndromes: investigations and management 252 Vertigo, dizziness, and unsteadiness: introduction 254 Dizziness, unsteadiness, or off balance: neurological causes 256 Benign paroxysmal positional vertigo (BPPV) 258 Dizzyness, unsteadiness, or off balance: non-neurological causes 262 Neurogenetic disorders: introduction 264 Hereditary ataxias 266 Genetic neuropathies 268 Inherited myopathies 272 Myotonic dystrophy (MD) 276

33. DETAILED CONTENTS1 xxix Inherited movement disorders 278 Inherited mitochondrial disorders 280 Inherited dementias 282 Inherited neurocutaneous syndromes 284 Hereditary metabolic diseases 286 Infectious disease: bacterial meningitis 288 Viral encephalitis 292 Neurology of HIV/AIDS: introduction 296 Neurological disorders due to HIV 298 Opportunistic infections associated with HIV 300 MRI images in infectious diseases 304 Lumbar puncture 308 Intravenous immunoglobulin (IV Ig) 312 Diagnosis of brainstem death 314 5 Neurosurgery 315 Subarachnoid haemorrhage (SAH) 316 Imaging of SAH: examples 320 Spontaneous intracranial haemorrhage (ICH) 328 Imaging of ICH: examples 330 Cerebral aneurysms 334 Cerebral arteriovenous malformations (AVM) 338 Cavernous haemangioma (cavernoma) and developmental venous anomaly (DVA) 340 Dural arteriovenous fistulae (dAVF) 342 Hydrocephalus 344 Complications of shunts 348 Intracranial tumours 350 Intracranial tumours: management of specific tumours 354 Imaging of intracranial tumours: examples 360

34. DETAILED CONTENTSxxx Imaging of spinal tumours: examples 372 Head injuries (HI) 376 Management of specific head injuries 380 Imaging of head injuries: examples 386 Spinal injuries 392 Imaging spinal injuries: examples 396 Degenerative spinal conditions: cervical spine 402 Degenerative spinal conditions: thoracic and lumbar spine 406 Imaging of degenerative spinal conditions: examples 410 Developmental abnormalities 414 Imaging of developmental abnormalities: examples 418 Syringomyelia 422 6 Clinical neurophysiology 425 Introduction 426 Electroencephalography (EEG): introduction 428 EEG: use and abuse 430 EEG: abnormal rhythms 432 EEG and epilepsy 436 EEG and diffuse cerebral dysfunction 440 EEG in the intensive care unit 444 Technical summary of nerve conduction studies (NCS) 446 Peripheral nerve disorders: NCS abnormalities 450 Technical summary of needle electromyography (EMG) 452 Needle EMG: patterns of abnormality 454 NCS and needle EMG findings in myopathies and motor axonal loss 460

35. DETAILED CONTENTS1 xxxi NCS and needle EMG findings in GuillainBarr syndrome 462 NCS and needle EMG findings in neuromuscular transmission disorders 464 Visual evoked responses (VERs) 468 Somatosensory evoked potentials (SSEPs) 470 Brainstem auditory evoked responses (BAERs) 474 Normal values in clinical neurophysiology 476 7 Neuroradiology 479 Techniques in diagnostic radiology 480 Cerebrovascular disease 486 Subarachnoid haemorrhage 490 Intracerebral haemorrhage 492 Guidelines for head injury 494 Imaging strategies for cervical spine trauma 496 Guidelines for the neuroradiology of tumours 498 CNS infections 500 Appendices 505 1 Neurological disability scales 507 2 Clinical pearls 511 3 Neurological eponyms 515 4 Useful websites 523 Index 525 Detailed contents

37. Chapter 1 1 Neurological history and examination Principles of neurological history taking 2 The general examination 4 Cranial nerve 1 (olfactory nerve) 6 Cranial nerve 2 (optic nerve and visual pathway) 8 Cranial nerves 3 (oculomotor), 4 (trochlear), and 6 (abducens) 12 Cranial nerves 5 and 712 16 Examination of the upper and lower limbs 18 Bedside cognitive testing, including language 24 The mini-mental state examination (MMSE) 30

38. CHAPTER 1 Neurological history and examination2 Principles of neurological history taking The primary role of the examination becomes the testing of the hypotheses derived from the history (William Landau) The usual approach to a clinical problem is to ask the following: - Where is the lesion, e.g. brain, spinal cord, anterior horn cell, peripheral nerve, neuromuscular junction, muscle? - What is the aetiology, e.g. vascular, degenerative, toxic, infective genetic, inflammatory, neoplastic, functional? - What is the differential diagnosis? - Is treatment possible? - What is the prognosis? A detailed history usually will yield more information than the neurological examination and ancillary tests. - Family members and eyewitness accounts are essential, e.g in patients with dementia and blackouts. Obtain a history by telephone if necessary. - A review of the case notes if available is very useful. - Analysis of symptoms will follow a similar plan: - date/week/month/year of onset; - character and severity; - location and radiation; - time course; - associated symptoms; - aggravating and alleviating factors; - previous treatments; - remissions and relapses. Past medical history Do not always accept the patients diagnostic termsenquire into specific symptoms, e.g. migraine, seizure, stroke. Family history Draw a family tree. Document specific illnesses and cause of death if known. In certain communities enquire about consanguinity. Social history This should include: - alcohol; - smoking; - recreational drug use; - risk factors for HIV; - detailed travel history; - dietary habits.

40. CHAPTER 1 Neurological history and examination4 The general examination This starts on first meeting the patientit is useful practice to collect patients from the waiting room. - Assess gaitbroad-based, unsteady, reduced arm swing on one side? - Look for tremormay only be evident when walking. - Look for loss of facial expression. - Assess speechdysarthria General examination is essential: ideally all patients should be stripped to the underclothes. - Cardiovascular system. Pulse, heart sounds, blood pressure (lying down and standing after 3 minutes if any suggestion of autonomic involvement). - Respiratory system. Diaphragmatic movement. May need to measure forced vital capacity (FVC) not FEV1 in, e.g. GBS, MG. - Gastrointestinal system. Palpate for hepatosplenomegaly or abdominal masses. - Genitalia. In men testicular examination should be considered. PR examination if malignancy suspected or assessment of anal tone and sensation if cord or cauda equina compression in differential diagnosis. - Breasts. Essential if neoplastic or paraneoplastic conditions are considered. - Examine the spinehairy patch may indicate underlying spinal disorder or a dermal sinus. Auscultation over spine may reveal the bruit of a dural AVM. - Skinmelanoma. Vitiligo indicating underlying autoimmune disorder, e.g. MG. - Head. Remember to palpate the temporal arteries in elderly headache patients; auscultation may reveal a bruit. Palpate the trapezii for evidence of tenderness in muscle tension and cervicogenic headache.

42. CHAPTER 1 Neurological history and examination6 Cranial nerve 1 (olfactory nerve) - Patients may not recognize a problem unless it is essential for work or hobbies, e.g. chef. Therefore question specifically. - History may indicate local nasal or sinus disease, preceding URTI or head injury. - Nose is supplied by the olfactory and trigeminal nerves. Irritants like NH3 stimulate the trigeminal nerve and may be misleading. - Use the University of Pennsylvania Smell Identification Test (UPSIT) if available. Otherwise use bedside products, e.g. orange peel, coffee, chocolate. Ask if there is a smell (perception, peripheral process) and then identify it (cognitive, central process). - Anosmia commonly occurs after viral infections and head injury. - In idiopathic Parkinsons disease (80%) and Alzheimers disease, loss of sense of smell may be an additional early feature. - Other causes of anosmia: - Refsums disease; - olfactory groove meningioma; - superficial siderosis; - Kallmans syndrome (anosmia + hypogonadism, X-linked recessive); - paraneoplastic disorders.

44. CHAPTER 1 Neurological history and examination8 Cranial nerve 2 (optic nerve and visual pathway) Visual acuity (VA) - Distance VA of each eye is tested with the Snellen chart. This compares what a normal person can see at 6 m. Below the age of 40 years most should see better than 6/6. In older patients VA < 6/9 needs explanation. - Correction for refractive errors with glasses or using a pinhole. - Near VA assessed with Jaeger reading charts. - In papilloedema VA preserved unless chronic. In optic neuritis or infiltration VA impaired. - Colour vision tested with Ishihara colour plates. Visual field - Visual field is assessed by confrontation with each eye in turn using a red pin (5 mm red target). Finger waving is too crude. - Goldmann perimeter is a bowl-shaped device and uses small light targets (kinetic). - Humphrey is an automated technique (static). - Visual inattention indicates parietal lobe dysfunction. - Uncooperative or aphasic patientsobserve reaction to menace. Visual field defects - Monocular field defect: ocular, retinal, or optic nerve disorders. - Constricted fieldsglaucoma, chronic papilloedema. - Tunnel visionretinitis pigmentosa. - Tubular visionnon-organic. - Central scotomaoptic nerve or macular disease. - Altitudinal defects are due to retinal vascular lesions as no vessels cross the horizontal raphe. Defects affecting both eyes may indicate a lesion of or behind the optic chiasm (vertical meridian). The common patterns of field loss are shown in Table 1.1. Fig. 1.1 shows a diagram of visual field defects.

45. CRANIAL NERVE 2 (OPTIC NERVE AND VISUAL PATHWAY)1 9 Left Right Optic chiasm Optic radiation Optic tract Optic nerve Lateral geniculate body 1 2 3 4 5 6 Left eye Right eye Defects in visual field of 1 2 3 4 5 6 Fig. 1.1 Diagram of visual field defects. 1. Unilateral blindness, 2. Bitemporal hemianopia, 3. Homonymous hemianopia, 4. Superior quadrantanopia; 5, 6. Inferior and superior quadrantanopias with macular sparing. Permission requested from Brown University, Rhode Island, USA. Table 1.1 Common patterns of visual field loss. Field defect Site of lesion(s) Aetiology Homonymous hemianopia Optic tract, optic radiation, occipital lobe Stroke, tumour Superior quadrantanopia Temporal lobe Stroke, tumour Inferior quadrantanopia Parietal lobe Stroke, tumour Bitemporal hemianopia Optic chiasm Pituitary adenoma, craniopharyngioma Binasal hemianopia Perichiasmal Bilateral internal carotid artery aneurysms Junctional scotoma Junction of optic nerve and chiasm Tumour Bilateral scotomas Occipital pole Head injury

46. CHAPTER 1 Neurological history and examination10 Clinical points - Complete homonymous hemianopia indicates only that the lesion is behind the optic chiasm. The more posterior the lesion, the more congruous the defect. - Macular sparing occurs because the middle cerebral artery supplies the occipital pole and the posterior cerebral artery the rest of the lobe. - Junctional lesions between the optic nerve and chiasm affect ipsilateral optic nerve fibres and fibres from the inferior nasal retina of the opposite optic nerve as they loop after decussation. Pupillary reactions - Test reaction to light: direct and consensual with a bright pen torch; ophthalmoscope light not strong enough. - Accommodation reflex is observed by watching the pupil as gaze is shifted from a distant object to a near object. - MarcusGunn pupil (afferent pupillary defect) results from optic nerve dysfunction or, if extensive, retinal disease. Detected by the swinging torch testa bright light is quickly moved back and forth between the eyes. The affected eye dilates rather than constricts when the light is swung to it because less light is perceived by the damaged pathway. Fundoscopy with the direct ophthalmoscope - confirm the red reflex and assess the clarity of the media; - assess disc colour for pallor. Fundoscopic findings - Pigmented temporal crescent seen in myopes. - 80% of normal discs will have venous pulsation. May be elicited by gentle eyeball pressure. - Papilloedema - hyperaemia of disc margin; - blurring of margins; - raised optic disc; - engorged veins; - haemorrhages; - cotton wool spots and exudates; - retinal folds. - Retinal abnormalities; - hard and soft exudates; - microaneurysms and new vessel formation; - pigmentary changes (bone spicules in retinitis pigmentosa); - macular changes (star, cherry red spot). - Drusen or hyaline bodies are shiny bodies on the surface, near or buried in the disc elevating it and resembling papilloedema. - Medullated nerve fibre layer (pearly white) is myelin from the optic nerve that continues into the nerve fibre layer. May be confused with papilloedema.

47. CRANIAL NERVE 2 (OPTIC NERVE AND VISUAL PATHWAY)1 11 Table 1.2 Pupillary abnormalities Abnormality Pupils Other features Tests 3rd nerve palsy Dilated; no response to light or accommodation Weakness: MR, IO, IR, SR. Ptosis (complete/partial) Horners syndrome (meiosis, ptosis, enophthalmos, anhidrosis) Constricted pupil; reacts to light and accommodation Partial ptosis, also upside- down ptosis (lower lid elevation), anhidrosis, enophthalmos 10% cocaine dilates normal pupil but not sympathetic denervated one.1% hydroxyampheta- mine dilates pupil in first or second order neuron damage. Argyll Robertson pupil Small, horizontally elongated pupil. Response to accommodation but not to light Syphilis, diabetes Tonic pupil (Adie). Usually unilateral Dilated pupil constricts slowly to accomodation. Unreactive to light but will constrict on prolonged and intense illumination. Vermiform movements visible on slit lamp Generalized areflexia = HolmesAdie syndrome 0.125% pilocarpine constricts pupil

48. CHAPTER 1 Neurological history and examination12 Cranial nerves 3, 4, and 6 Cranial nerves 3 (oculomotor), 4 (trochlear), and 6 (abducens) Figure 1.2 shows the muscles innervated by cranial nerves 3, 4, and 6. Extra-ocular eye movements - Monocular diplopia due to refractive error, cataract, media opacity, macular disease, visual cortex disorder (bilateral) or functional. - Horizontal diplopia is due to weakness of medial or lateral rectus. - Oblique separation with one image slightly tilted is due to superior or inferior oblique weakness. - Images are maximally separated when direction of gaze is towards the site of maximal action of the paretic muscle. - The outer image comes from the paretic eye. Eye movements: pursuit and saccadic - Fixationobserve the fixed eye for 30 seconds: horizontal square wave jerks (SWJ) seen in cerebellar disease, PSP, and MSA. - Saccades (rapid conjugate eye movements) tested by asking the patient to fixate between two targets (fist right hand and fingers left hand). - Observe for speed of initiation (latency). - Saccadic velocity. - Accuracy. (Undershoot = hypometria found in cerebellar disorders, PD and HD. Overshoot = hypermetria caused by cerebellar dysfunction.) - Helps detect subtle internuclear ophthalmoplegia (INO)lesion of medial longitudinal fasciculus. In a partial lesion, slowing of adduction ipsilateral to the lesion and nystagmus in contralateral abducting eye. In complete lesion adduction absent. Causes: demyelination or vascular. See Fig. 1.3. - Smooth pursuit. Test horizontal and vertical movements by tracking a target keeping the head still. Broken pursuit non-specific sign due to cerebellar disease, drugs, e.g. anticonvulsants and sedatives. If only in one direction indicates posterior cortical lesion ipsilateral to the direction of broken pursuit.

49. CRANIAL NERVES 3, 4, AND 61 13 SR IR IR LR LR MR MR IO SO SO SRIO Left eye Right eye Fig. 1.2 Diagram showing muscles innervated by cranial nerves 3, 4, and 6, Cranial nerve 3: medical rectus (MR); inferior oblique (IO); superior rectus (SR); inferior rectus (IR). Cranial nerve 4; superior oblique (SO). Cranial nerve 6: lateral rectus (LR). VI Pons Medial longitudinal fasciculus IIIMidbrain Lateral rectus Medial rectus Horizontal eye movements Fig. 1.3 Horizontal eye movements

50. CHAPTER 1 Neurological history and examination14 Nystagmus - Involuntary oscillation is initiated by a slow drift of the eye. If followed by a corrective fast phase = jerk nystagmus; if both phases have equal velocity = pendular nystagmus. Direction of nystagmus described by fast phase. - Jerk nystagmus due to vestibular damageperipheral (labyrinth, vestibular nerve) or central (brainstem). See Table 1.3. Other types of jerk nystagmus (central vestibular) - Downbeat nystagmus: - present in the primary position; - accentuated on lateral gaze; - due to disturbance of vestibulocerebellum caused by ArnoldChiari malformation, cerebellar degeneration, drug toxicity, e.g. lithium. - Upbeat nystagmus: - present in primary position; - due to lesion in the tegmental grey matter of brainstem; - causes: MS, vascular, cerebellar degeneration. - Gaze-evoked nystagmus (GEN): - only present on eccentric gaze not primary position; - may be horizontal, upbeating on upgaze and or down beating on downgaze; - bilateral horizontal GEN due to cerebellar and brainstem disorders, drugs, alcohol, diffuse metabolic disorders.

51. CRANIAL NERVES 3, 4, AND 61 15 Table 1.3 Features of peripheral and central vestibular nystagmus Peripheral Central Unidirectional fast phase beating away from affected labyrinth Uni- or multidirectional Associated with severe vertigo, vomiting, nausea Mild symptoms. Other neurological signs, e.g. disconjugate eye movements, pyramidal signs Amplitude increases with gaze towards the direction of the fast phase May be gaze-evoked Various componentshorizontal, torsional, vertical Suppressed by fixation (Freznel goggles removes fixation) No change with fixation

52. CHAPTER 1 Neurological history and examination16 Cranial nerves 5 and 712 Cranial nerve 5 (trigeminal) Sensory via three divisions (ophthalmic V 1 , maxillary V 2 , mandibular V 3 ). - Ophthalmic (V 1 ). Extends posteriorly to the vertex. - Sensation but not taste to anterior 2/3 of the tongue also supplied by TG nerve. - Motor fibres to muscles of mastication (temporalis, masseter, and pterygoids via mandibular division). - Jaw deviates to side of weak pterygoid muscle. - Corneal reflex has a consensual component. Useful in the presence of an ipsilateral facial palsy. - Jaw jerkif brisk indicates pathology above midbrain level. - Rogers sign = numb chin syndrome due to metastatic deposit around inferior alveolar branch. Breast cancer, lymphoma. Cranial nerve 7 (facial) - Supplies the muscles of facial expression and taste to anterior two thirds of the tongue (via corda tympani branch). - Lower motor neuron facial palsies result in complete ipsilateral facial weakness. - The upper face is bilaterally innervatedfrontalis and to a lesser extent orbicularis oculi are spared in upper motor neuron palsies. Cranial nerve 8 (acoustic nerve) - Two divisions: - cochlear (hearing); - vestibular (balance). - Hearing is crudely tested by whispering numbers in one ear whilst blocking the other. - Rinnes test256 Hz tuning fork first held in front of the external auditory meatus and then placed firmly on the mastoid. - Normal (positive test), air conduction louder > bone conduction. - Conductive deafness, BC > AC. - Sensorineural deafness, Rinnes positive. - Webers lateralization testtuning fork placed in middle of forehead. - Unilateral conductive deafnesslouder to the ipsilateral side. - Sensorineural deafnesslouder to contralateral side. - Vestibular function tested using: - Hallpikes test (see Fig. 4.20 in Benign paraoxysmal positional vertigo, Chapter 4). - Unterbergers testwith eyes closed and arms extended, patient marches on the spot for one minute. Positive test if veers to one side. (Does not differentiate central from peripheral.)

53. CRANIAL NERVES 5 AND 7121 17 Cranial nerve 9 (glossopharnygeal nerve) - Taste fibres from posterior third of the tongue. - General sensation tympanic membrane, mucous membranes from posterior pharynx, tonsils, and soft palate. - Afferent part of the gag reflex. Cranial nerve 10 (vagus nerve) - Motor fibres innervate the striated muscles of palate, pharynx, larynx. - Soft palate observed as patient says aahh. - Deviation away from side of lesion. - Lesions of recurrent laryngeal branch cause ipsilateral vocal cord paralysis with dysphonia and a weak cough. - Parasympathetic autonomic fibres travel in the vagus nerve to the respiratory, GI, and cardiovascular systems. Cranial nerve 11 (accessory nerve) - Innervation to sternocleidomastoid (SCM) and trapezius. - SCM (supplied by ipsilateral hemisphere) assessed by asking patient to twist the head against resistance and palpate contralateral SCM. - Trapezius assessed by shoulder shrug and palpating muscle. Cranial 12 (hypoglossal nerve) - Observe for fasciculationsmay be difficult. Observe with tongue inside the mouth. - Tongue strength assessed by asking patient to push inside the mouth against cheek. - Tongue movement dexterity assessed by asking patient to move tongue side to side. Slowness without wasting suggests spasticity. - In LMN lesions tongue deviated to the side of the lesion.

54. CHAPTER 1 Neurological history and examination18 Examination of the upper and lower limbs Ideally, patient should be stripped to underclothes. General points - Document hand dominance. - Look for wastingfirst dorsal interosseus muscle easiest (ulnar). - Examine scapular muscles (winging of the scapula due to lesions of long thoracic nerve). - Palpate extensor digitorum brevis (EDB) on the foot. - Observe for fasciculationmay need to spend a few minutes in good light. - Screening testask patient to hold arms outstetched palms up with eyes closed. - Pronator drift indicates mild pyramidal weakness. - Pseudoathetosis (involuntary movements of fingers) indicates loss of position sense. - Postural tremor may be caused by essential tremor, demyelinating neuropathy, or drugs (sodium valproate, steroids). Tone i Spastic (pyramidal) assessed by the following: - Upper limbs: - rapid flexion/extension movement at the elbow (clasp knife); - supinator catch (rapid supination movement at wrist); - Hoffmans sign (rapid flexion at DIPJ of middle finger results in brisk flexion movements at other fingers)-positive in upper motor lesions. - Lower limbs: - a brisk flick at the knee when legs extended results in a catch if tone increased; - test for clonus at ankles. i Extrapyramidal increase in tone assessed: - by slow flexion/extension movments at the wrist; - may be enhanced by synkinesia (ask patient to move contralateral limb). Muscle strength All that is required is maximal strength for one seconduseful in patients with giveway weakness. Table 1.4 gives the muscles to be tested and Table 1.5 gives a grading system to evaluate the results.

55. EXAMINATION OF THE UPPER AND LOWER LIMBS1 19 Table 1.4 Important myotomes Muscle* Roots Nerve Action Trapezius C3, 4 Spinal accessory Shrug shoulder Rhomboids C4, 5 Dorsal scapular Brace shoulders back Supraspinatus C5, 6 Suprascapular Abduct shoulder 15o Deltoid C5, 6 Axillary Abduct shoulder 1590 Infraspinatus C5, 6 Suprascapular External rotation of arm Biceps C5, 6 Musculocutaneous Flex forearm Triceps C6, 7 Radial Extend forearm Extensor carpi C5, 6 Radial Extend wrist Finger extensors C7, 8 Posterior interosseous Extend fingers FDP I and II C8, T1 Median Flex DIPJ FDP III and IV C8, T1 Ulnar Flex DIPJ FDS C8, T1 Median Flex PIPJ APB C8, T1 Median Abduct thumb OP C8, T1 Median Thumb to 5th finger ADM C8, T1 Ulnar Abduct 5th finger 1ST DIO C8, T1 Ulnar Abduct index finger Iliopsoas L1, 2 Femoral Flex hip Hip adductors L2, 3 Obturator Adduct hip Hip extensors L5, S1 Inferior gluteal Extend hip Quadriceps L2, 3 Femoral Extend knee Hamstrings L5, S1 Sciatic Flex knee Tibialis anterior L5, S1 Deep peroneal Dorsiflex foot Gastrocnemius S1, 2 Tibial Plantarflex foot Tibialis posterior L4, 5 Tibial Invert foot EHL L5, S1 Deep peroneal Dorsiflex hallux Peroneus longus L5, S1 Superficial peroneal Evert foot * Muscles in bold font are essential in a basic neurological examination. Table 1.5 MRC grading system for muscle strength MRC grade Observed muscle power 0 No movement 1 Flicker of movement 2 Movement with gravity eliminated 3 Movement against gravity 4, 4+, or 4 Weak 5 Normal power

56. CHAPTER 1 Neurological history and examination20 Coordination Upper limbs - Finger nose testing: intention tremor with increased amplitude near target. - Dysdiadokinesia (rapid pronation/supination movements of one hand on the palm of contralateral hand. - Tapping to elicit rhythm. Lower limbs - Heel/shin testing. - With eyes open and closed to assess for sensory ataxia (worse). Sensory testing - Do not spend too much time on this. - Map out abnormality for pain (pin prick), light touch (cotton wool), vibration (128 Hz tuning fork), joint position (at DIPJ) in fingers and toes and working proximally. Figure 1.4 shows the dermatomes of the upper and lower limbs. C6 AXIAL LINES: ventral; dorsal C3 (a) C6 C4 C5 T2 T1 T1 C7 C8 C7 C8 C5 T2 C4 C3 Fig. 1.4 (a) Dermatomes of upper limb.

57. EXAMINATION OF THE UPPER AND LOWER LIMBS1 21 Anterior axial line Posterior axial line T12 L1 (b) L3 L4 L5 L2 S2S3 L2S4 S2 L3 L4 S1 L5 L4 L5 L3 S1 S3/4 L1 Fig. 1.4 (b) Dermatomes of lower limb. In both diagrams note the axial lines. Reproduced with permission from MacKinnon, P. and Morris, J. (2005). Oxford Textbook of Functional Anatomy, Vol. 1, 2nd edn. Oxford University Press, Oxford.

58. CHAPTER 1 Neurological history and examination22 Deep and superficial tendon reflexes (see Table 1.6) Deep tendon reflexes - The deep tendon reflexes are graded from 0 (absent), (present with reinforcement), + (depressed), ++ (normal), +++ (increased). - Reinforcement can be obtained by jaw clenching or Jendrassiks manoeuvre (patient links hands and pulls). - Deep tendon reflexes may also be invertedthe tested reflex is absent but there is spread to a lower level. This indicates a lower motor neuron lesion at the level of the reflex but an upper motor neuron lesion below (most common at C5/C6). Main superficial reflexes - Abdominal (upperT8/9; lowerT10/11)absent in some UMN lesions. - Cremasteric (L1/2)elicited by stroking inner thigh with reflex ipsilateral testicular elevation. - Anal (S4/5)scratch anal margin with reflex contraction visible. Gait examination - Rombergs sign. Patient standing with eyes open. On closure of eyes, swaying or fall suggesting disturbance of proprioception. Useful in non-organic disorders. The various gait disturbances encountered in clinical practice are shown in Table 1.7.

59. EXAMINATION OF THE UPPER AND LOWER LIMBS1 23 Table 1.6 Deep tendon reflexes Reflex Nerve Root Biceps Musculocutaneous C5/6 Supinator Radial C5/6 Triceps Radial C7 Finger flexors Median/ulnar C8 Knee Femoral L3/4 Ankle Tibial S1/2 Table 1.7 Gait disturbances encountered in clinical practice Gait disturbance Description Common causes Gait apraxia Small shuffling steps marche petits pas; difficulty in starting to walk; cycling on bed significantly better Small vessel disease, hydrocephalus Parkinsonian Shuffling; loss of arm swing Parkinsonism Spastic paraparesis Stiff walking through mud Cord lesion, parasaggital lesion Myopathic Waddling Myopathic, dystrophic disorders Foot drop Foot slapping Neuropathy, radiculopathy rarely UMN Cerebellar ataxia Wide-based; drunken Any cerebellar pathology Sensory ataxia Wide-based; foot slapping; deteriorates with eye closure Neuropathy, subacute combined degeration of cord, posterior column disorders, e.g. MS

60. CHAPTER 1 Neurological history and examination24 Bedside cognitive testing, including language There is no point in attempting a cognitive assessment in a patient who is drowsy or uncooperative. 1 Alertness Record the level of wakefulness and reactivity. 2 Orientation - Time (time of day; day of the week, month, and year). Disorientation in time common in delirium, moderate dementia, and amnestic syndromes. - Place (building, town, county, country). - Person (name, age, date of birth). Dysphasic patients may appear confused due to an inability to understand or express themselves. 3 Attention and concentration - Count backwards from 20. - Months of the year backwards. - Digit span. Ask patient to repeat string of increasing digitstwo trials at each level. Record highest level at which either trial correct, e.g. 3 4 8 4 7 9 2 3 6 7 1 4 5 9 2 7 9 5 6 1 8 7 2 3 Normal 6 1 4 Memory Anterograde memory - Name and address, e.g. John Green, 157, Church Lane, Cambridge. - Assess immediate recall and after 5 minutes. Retrograde memory - Dates for Second World War. - Recent world eventssports, royal family news, prime minister. - Autobiographical memoryparents, childhood events.

61. BEDSIDE COGNITIVE TESTING, INCLUDING LANGUAGE1 25 5 Frontal executive function (frontal lobe) Initiationverbal fluency test - Ask patient to generate as many words as possible in 1 minute beginning with the letter F, A, or S, excluding names of people or places. Normal: 15 depending on age and intellect. - Name as many animals or fruit in 1 minute. > 20, normal; < 10 abnormal. Abstract thought Interpretation of proverbs (frontal lobe disorders result in concrete interpretations), e.g. a stitch in time saves nine; too many cooks spoil the broth. Cognitive estimates Frontal patients give bizarre and illogical answers to questions like the following: - How many camels are there in Holland? - What is the height of an average English woman? - What is the population of London? Alternating hand movements - With arms out, fingers of one hand extended; the other with fist clenched. Reverse positions rhythmically. See Figure 1.5. - Luria 3 step test. See Figure 1.6. Difficulties with complex motor movements associated with left frontal lesions.

62. CHAPTER 1 Neurological history and examination26 6 Dominant (usually left) hemisphere function Language Aphasia (Table 1.8) and dysphasia are impairments of language function. Dysarthria is the abnormal motor production of speech. - Spontaneous speech assessed during conversation and description of a picture. - Articulation (abnormal in bulbar, cerebellar, and basal ganglia disorders). - Fluencyin-non-fluent speech reduced rate of word production and short phrases. - Grammarlack of pronouns, prepositions, and errors of tense. Correlates with non-fluent language. - Paraphasic errorsword substitution, e.g. black for blank (similar sounding = phonemic) or apple for pear (meaning-based = semantic). - Prosodyloss of intonation, pitch, and stress occur in right hemisphere lesions but also in non fluent speech and in articulatory disorders. - Naming. Record 10 itemsa mixture of common and uncommon objects, e.g. pen, watch, sleeve, watch winder, buckle. - Comprehension: - single wordspoint to objects in the room, e.g. door, ceiling - complex instructionspick up the piece of paper, fold it in half and give it to me - conceptualwhat is the colour of a banana? What is the name of item in the kitchen that enables you to cut? - Repetition, e.g. the band played and the audience clapped, no ifs, ands, or buts. - Reading a passage (see example in box) usually parallels spoken language problems. Occasionally alexia can occur without aphasia. - Writingask patient to write any novel sentence. Dictate a sentence e.g. the cat sat on the mat. Calculation Simple arithmetic (addition, subtraction). Praxis skills First to command and, if not possible, then by imitation show me how you would: - blow a kiss (buccofacial); - wave goodbye (limb gestures); - hammer a nail (object use). Table 1.8 Types of aphasias and characteristics Type of aphasia Fluency Repetition Comprehension Naming Brocas (inferior frontal lobe) Non fluent Affected Not affected Affected Wernickes (posterior superior temporal lobe) Fluent Affected Affected Affected

63. BEDSIDE COGNITIVE TESTING, INCLUDING LANGUAGE1 27 Fig. 1.5 Alternating hand movements test. The hand positions (above) and the sequence of movements to the patient (below) are shown. Fig. 1.6 Luria three-step test sequence of hand positions (firstedgepalm) is shown. Example of passage for reading On an early autumn Monday morning, Dr David Gordon, driving his Mercedes convertible, reflected upon his weekend that he had spent relaxing at their seaside cottage in Aldeburgh on the Suffolk coast. As a busy general practitioner in Peckham, his morning surgery consisted of the usual mixture of patients with headaches, coughs and colds and intractable social problems. Lunch, as always, was of a cheese baguette accompanied by a yogurt drink. Driving home exhausted but fulfilled, he looked forward to a quiet supper with his wife Rachel followed by watching Coronation Street on the TV.

64. CHAPTER 1 Neurological history and examination28 7 Non-dominant (usually right) hemisphere function Neglect - Sensory neglect: patient ignores visual, tactile, and auditory stimuli from left side. - Sensory extinction: patient responds to visual or tactile stimulus from each side separately but, when bilateral stimuli presented ignores neglected side. - Hemispatial neglect: in drawing a clock face, one side of clock is omitted. (see Fig 1.7). - Dressing apraxia: patient unable to dress, e.g. shirt inside out. - Constructional ability. Copy shapes, e.g. overlapping pentagons. (see Fig 1.8) - Prosopagnosia: impaired facial recognition.

65. BEDSIDE COGNITIVE TESTING, INCLUDING LANGUAGE1 29 1 2 3 4 5 12 6 Fig. 1.7 In drawing a clock face patient with hemispatial neglect will omit one side. Fig. 1.8 Overlapping pentagons from the Mini-Mental State examination.

66. CHAPTER 1 Neurological history and examination30 The mini mental state examination(MMSE) The mini-mental state examination (MMSE) Commonly used bedside test (Table 1.9) 1 . Caveats include: - take into account age, education, culture; - insensitive to focal deficits especially frontal lobe; - cut-off score 24/30 but patients with superior background IQ may perform well despite significant cognitive impairment. 1 Folstein, M.F., Folstein, S., and McHugh, P.R. (1975), mini-mental state: a practical method for grading the cognitive state of patients for the clinician. J. Psychiatric Res. 12, 18998.

67. THE MINI MENTAL STATE EXAMINATION (MMSE)1 31 Table 1.9 The Mini-Mental state examination (MMSE)* Test Score per Item Maximum score/test Orientation Year, month, day, date, season Country, county, town, hospital , ward/room 1 1 5 5 Registration Examiner names 3 objects (e.g. ball, pen, key); Patient repeats each item 1 3 Attention Ask patient to start with 100 and subtract 7. Stop after 5 subtractions, e.g. 100, 93, 86, 79, 72, 65 or Ask patient to spell 5-letter word backwards, e.g. world. Score number of letters in correct order 1 5 Recall Ask for the 3 words you asked patient to number in Registration test 1 3 Language Naming: point to object and ask patient to name it. e.g. watch, tie 1 2 Repetition Ask patient to repeat sentence after you (only 1 trial allowed), e.g. no ifs, ands, or buts 1 1 3-Stage command e.g. take this paper, fold it in half, and give it to me. Score 1 point for each stage of command correctly executed 1 3 Reading Ask patient to read a command on paper, e.g. close your eyes, and to execute it 1 1 Writing Ask patient to write a sentence. To score 1 it must be sensible and must contain a noun and a verb 1 1 Copying Copy picture of intersecting pentagons (Fig. 1.8). To score 1, all 10 angles must be present and two must intersect 1 1 Maximum possible score 30 * No half-points are given in the MMSE. Home or hospital depending on location of the test.

69. Chapter 2 33 Neuroanatomy The cranial cavity 34 Dermatomes of the upper and lower limbs 36 Innervation of the upper limbs 38 Innervation of the lower limbs 46 Cross-sections of the brain and spinal cord 52

70. CHAPTER2Neuroanatomy34 Thecranialcavity Pituitary stalk Superior ophthalmic vein Sphenoparietal sinus Cavernous sinus Middle meningeal artery Sigmoid sinus Basilar artery Left vertebral artery Transverse sinus Foramen magnum Straight sinus XII XI X IX VIII VII VI Ophthalmic (Superior orbital fissure) (Foramen rotundum) (Foramen ovale) (Internal acoustic meatus) (Jugular foramen) (Hypoglossal canal) maxillary mandibular IV III II I Ophthalmic artery Internal carotid artery Trigeminal ganglion V Inferior and superior petrosal sinuses Fig. 2.1 Interior of skull base; vessels and nerves. Adapted with permission from MacKinnon, P. and Morris, J. (2005). Oxford Textbook of Functional Anatomy, Vol. 3, 2nd edn. Oxford University Press, Oxford.

72. CHAPTER 2 Neuroanatomy36 Dermatomes of the upper and lower limbs (a) (b) Fig. 2.2 Approximate distribution of dermatomes: (a) on the anterior aspect of the upper limb; (b) on the posterior aspect of the upper limb.

73. DERMATOMES OF THE UPPER AND LOWER LIMBS1 37 (c) (d) Fig. 2.2 Approximate distribution of dermatomes: (c) on the lower limb; (d) on the perineum. Reprinted from Aids to the Examination of the Peripheral Nervous System; 4th edn, (2000) pp. 569, with permission from Elseiver.

74. CHAPTER 2 Neuroanatomy38 Innervation of the upper limbs from C4 Medial cutaneous of arm and forearm Nerve to subclavius Long thoracic nerve (to serratus anterior) Musculo- cutaneous Medial pectoral nerve TRUNKS: Upper Medial CORDS: C5 NERVES: Axillary Nerve to rhomboids Post Ulnar C6 C7 C8 T1 Suprascapular nerve Lateral pectoral nerve Radial Nerves to subscapularis and latissimus dorsi Lower Middle Median Lateral ROOTS: Fig. 2.3 Brachial plexus: schematic diagram of trunks, cords, and branches. Reproduced with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2 nd edn. Oxford University Press, Oxford.

75. INNERVATION OF THE UPPER LIMBS1 39 Sensory Biceps Coraco- brachialis (a) Brachialis Sensory distribution (lateral cutaneous nerve of forearm) Biceps Coraco- brachialis (b) Brachialis Fig. 2.4 Course of musculocutaneous nerve. (a) Supply to muscles. (b) Supply to skin. Reproduced with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2 nd edn. Oxford University Press, Oxford.

76. CHAPTER 2 Neuroanatomy40 Pronators Palmar sensory Thenar muscles Flexors (except flexor carpi ulnaris and ulnar half of flexor digitorum profundus) Lateral 2 lumbricals Sensory Pronator teres Flexor carpi radialis Pronator quadratus Sensory distribution of digital branches Palmar sensory branch Flexor digitorum superficialis (b)(a) Anterior interosseous nerve Fig. 2.5 Course of median nerve. (a) Supply to muscles. (b) Supply to skin. Note: anterior interosseous nerve supplies flexor pollicis longus; flexor digitorum profundus to second and third digits, and pronator quadratus. Adapted with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2nd edn. Oxford University Press, Oxford.

77. INNERVATION OF THE UPPER LIMBS1 41 Sensory Flexor carpi ulnaris Flexor digitorum profundus (ulnar half) Most of the small muscles of the hand Dorsal branch (sensory) Hypothenar muscles Sensory Sensory Ulnar half of flexor digitorum profundus Flexor carpi ulnaris Sensory distribution (a) (b) Fig. 2.6 Course of ulnar nerve. (a) Supply to muscles. (b) Supply to skin. Reproduced with permission from MacKinnon, P. and Morris, J. (2005). Oxford Textbook of Func- tional Anatomy, Vol. 1, 2nd edn. Oxford University Press, Oxford.

78. CHAPTER 2 Neuroanatomy42 (a) Deltoid Brachio- radialis Deep (post interosseous branch) Sensory Teres minor Sensory Extensors Carpi radialis Supinator All the other extensors and abductor pollicis longus Carpal joints Triceps Latissimus dorsi Teres major Subscapularis Sensory Anconeus Sensory Posterior cutaneous n. of arm (b) Upper and lower lateral cutaneous n. of arm Posterior cutaneous n. of forearm Superficial branch of radial n. Fig. 2.7 (a) Posterior cord, axillary, and radial nerves: supply to muscles. Note: posterior interosseus branch supplies extensor digitorum communis, extensor pollicis longus, extensor carpi ulnars. (b) Course of radial nerve: supply to skin. Reproduced with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2 nd edn. Oxford University Press, Oxford.

79. INNERVATION OF THE UPPER LIMBS1 43 Axillary n. (circumflex) Lower lateral cutaneous n. of arm Posterior cutaneous n. of forearm Upper lateral cutaneous n. of arm Radial nerve Superficial br of radial n. Branches to superficial extensors Dorsal digital branches of radial n. Terminal branches to joints of carpus Posterior interosseous n. Posterior cutaneous n. of arm Suprascapular n. (from upper trunk) Superficial extensor tendons (cut) (c) Deltoid (reflected) Axillary (circumflex) nerve Teres minor Teres major Long head of triceps (d) (e) Fig. 2.7 (c) Course of radial nerve: sensory supply (to hand). (d) Course of axillary nerve. (e) Axillary nerve: supply to skin. Reproduced with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2 nd edn. Oxford University Press, Oxford.

80. CHAPTER 2 Neuroanatomy44 Medial cutaneous nerve of arm Ulnar nerve Medial cutaneous nerve of forearm Fig. 2.8 Distribution of medial cutaneous nerves of arm and forearm and of ulnar nerve. Reproduced with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2 nd edn. Oxford University Press, Oxford.

82. CHAPTER 2 Neuroanatomy46 Innervation of the lower limbs Lateral cutaneous of thigh To iliacus Femoral To gluteal muscles Sciatic Common peroneal (common fibular) Tibial To lateral rotators of hip Posterior cutaneous of thigh S3 S2 S1 L5 L4 L3 L2 Obturator To psoas Fig. 2.9 Lumbosacral plexus. Reproduced with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2 nd edn. Oxford University Press, Oxford.

83. INNERVATION OF THE LOWER LIMBS1 47 Iliacus (a) Femoral nerve Sartorius Rectus femoris Pectineus Vastus lateralis Vastus intermedius Vastus medialis Fig. 2.10 (a) Femoral nerve: supply to muscles. (b) Femoral nerve: supply to skin; also lateral cutaneous nerve of thigh. Reproduced with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2 nd edn. Oxford University Press, Oxford. Lateral cutaneous nerve of thigh Saphenous nerve Medial cutaneous nerve of thigh Intermediate cutaneous nerve of thigh (b)

84. CHAPTER 2 Neuroanatomy48 Obturator (a) nerve Gracilis Adductor brevis Obturator externus Adductor longus Adductor magnus Psoas (b) L,2,3,4 Fig. 2.11 Obturator nerve. (a) Supply to muscles. (b) Supply to skin. Reproduced with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2 nd edn. Oxford University Press, Oxford.

85. INNERVATION OF THE LOWER LIMBS1 49 Superior gluteal nerve (a) Inferior gluteal nerve Gluteus maximus Semitendinosus Semimembranosus Adductor magnus Tibial nerve Medial head of gastrocnemius Adductor hallucis Interossei Flexor digiti minimi Abductor digiti minimi Lateral plantar nerve Flexor hallucis longus Lateral head of gastrocnemius Common peroneal nerve Biceps femoris Sciatic nerve Tensor fasciae latae Gluteus minimus Gluteus medius Soleus Tibialis posterior Flexor digitorum longus Medial plantar nerve Abductor hallucis flexor hallucis brevis Flexor digitorum brevis Tibial nerve (b) Common peroneal nerve Lateral cutaneous nerve of calf Sural nerve Tibial nerve Lateral plantar nerve Medial plantar nerve Fig. 2.12 Sciatic and tibial nerves. (a) Supply to muscles. (b) Supply to skin. Reproduced with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2 nd edn. Oxford University Press, Oxford.

86. CHAPTER 2 Neuroanatomy50 Common peroneal nerve (a) Superficial peroneal nerve Peroneus longus Extensor digitorum brevis Peroneus brevis Peroneus tertius Extensor hallucis longus Extensor digitorum longus Tibialis anterior Deep peroneal nerve Lateral cutaneous nerve of calf (b) Superficial peroneal nerve Sural nerve Deep peroneal nerve Fig. 2.13 Common peroneal nerve. (a) Supply to muscles. (b) Supply to skin. Reproduced with permission from MacKinnon, P. and Morris, J. (2005) Oxford Textbook of Functional Anatomy, Vol. 1, 2 nd edn. Oxford University Press, Oxford.

88. CHAPTER 2 Neuroanatomy52 Cross-sections of the brain and spinal cord Oculomolor nerve Medial lemniscus and lateral spinothalamic tract Superior colliculus Aqueduct Red nucleus Substantia nigra Peduncle (pyramidal tract) Midbrain (cranial nerves 3 to 4 ) 3 3 Fig. 2.14 Cross-section, mid-brain. Cranial nerves 3 to 4. Medial longitudinal fasiculus Cranial nerve nuclei Medial lemniscus Motor fibres (corticobulbar and corticospinal) Lateral gaze centre Trigeminal nucleus Lateral spinothalamic tract Cerebellar fibres Pons (cranial nerves 5 to 8) 6 7 8 5 Fig. 2.15 Cross-section, pons. Cranial nerves 5 to 8.

89. CROSS-SECTIONS OF THE BRAIN AND SPINAL CORD1 53 Cerebellar tract (ipsilateral cerebellar signs) Medial lemniscus (contralateral position and vibration loss) Pyramidal tract (contralateral hemiplegia) Vestibular nucleus (vertigo, nausea, nystagmus) Cranial nerve nuclei Descending tract of V (ipsilateral loss of pinprick and temperature sense on the face) Descending sympathetic fibres Spinothalamic tract (contralateral pinprick and temperature sensory loss on the body) 4th Ventricle 12 12 10 9 Fig. 2.16 Cross-section, medulla. Cranial nerves 9 to 12. Ascending Spinal cord Descending 1 2 4 5 3 Fig. 2.17 Spinal cord cross-section. 1. Dorsal columns; 2. spinothalamic tracts; 3. corticospinal tracts; 4. anterior horn cells; 5. anterior spinal artery.

91. Chapter 3 55 Common clinical presentations Loss of consciousness 56 Acute vertigo 58 Acute headache (thunderclap headache) 62 Acute neuromuscular weakness 64 Acute focal neurological syndromes 68 Spastic paraparesis 70 Ataxia 72 Acute visual failure 76 Coma 80 Coma prognosis 84 Excessive daytime sleepiness 86 Tremor 90 Tics 94 Chorea and athetosis 96 Myoclonus 98 Dystonia 100

92. CHAPTER 3 Common clinical presentations56 Loss of consciousness This is a common problem. - Eyewitness account (verbal or written) is essential. May need to contact by telephone. - Better to label an episode ?Cause than diagnose epilepsy if not sure. Time will make the diagnosis clear. Avoid trials of anticonvulsants. - Advise the patient about implications for driving. May need to inform the DVLA. Aetiology Neurological causes - Epileptic seizures. 1 - Raised ICP (tumour especially posterior fossa lesions, hydrocephalus due to, e.g.colloid cyst). - SAH. - Sleep disorders (narcolepsy, cataplexy). - Basilar artery migraine (rare). - Cerebrovascular disease (rare, unless massive stroke or brainstem). Neurally mediated syncope - Neurocardiogenic (vasovagal) syncope. 1 - Carotid sinus syncope. - Situational syncope (cough, micturition). Cardiac syncope - Cardiac arrhythmias. 1 - Structural cardiopulmonary disease (aortic stenosis, HOCM, pulmonary embolus). Orthostatic or postural hypotension - Drugs 1 , e.g. vasodilators, antidepressants, L-dopa preparations. - Autonomic neuropathy (GBS, diabetes, amyloid). - Autonomic failure (MSA, PD). - Addisons disease. Metabolic disorders - Hypoglycaemia. - Hyperventilation induced alkalosis. Psychiatric disorders - Psychogenic non epileptic attacks. 1 1 Commonest causes.

93. LOSS OF CONSCIOUSNESS1 57 Diagnosis See Table 3.1. The eyewitness account will help make the diagnosis. Table 3.1 Features differentiating vasovagal syncope from epileptic seizures Seizure Syncope Trigger Rareflashing lights, hyperventilation Common (blood, needles, hot environment, standing, pain) Prodrome Commonauras Very commonnausea, lightheadedness, tinnitus, greying vision Onset Sudden Gradual Duration 13 min 130 sec Convulsive jerks Commonprolonged Commonbrief Incontinence Common Uncommon Tongue biting Common Rare Post-event confusion Common Rare Colour Pale, cyanotic (tonicclonic seizures) Very pale Investigations Consider the following: - Blood: hb (anaemia), glucose (especially in diabetics or if preprandial), K + , Ca 2+ . - ECG: 24-h tape (repeated) or, if necessary, a prolonged cardiomemo; echocardiogram for cardiac syncope. - Tilt table testing. Sensitive for syncopal tendencies. - EEG by itself does not diagnose epilepsy. 50% false negative rate for interictal EEG in patients with epilepsy. Reduced to 30% by repeating and 20% with sleep-deprived EEG. False positive EEG in up to 2% healthy young adults. - Ictal video-EEG-telemetry most sensitive and specific test for epilepsy. Note: frontal lobe epileptic seizures may appear normal even on ictal EEG. - Imagingpreferably with MRIfor focal seizures, focal signs, or signs and symptoms of iICP.

94. CHAPTER 3 Common clinical presentations58 Acute vertigo Vertigo is the illusion of rotation caused by asymmetry of neural activity between the right and left vestibular nuclei. Bilateral damage does not cause vertigo. Essential to determine if the vertigo is central or peripheral since cerebellar infarction/haemorrhage can be life-threatening and require neurosurgical intervention. Aetiology - Acute vertigo with no other symptoms or signs is very unlikely to be due to vertebrobasilar ischaemia. - Acute vestibular neuritis, presumed viral, affects lateral semicircular canal function. - Infarction within the territory of the anterior vestibular artery, a branch of the internal auditory artery, that in turn branches from the anterior inferior cerebellar artery. Clinical presentation is similar to that of a vestibular neuritis but usually occurs in older patients with risk factors for stroke such as diabetes, hypertension, and cardiac disease, e.g. atrial fibrillation. - Brainstem stroke, accompanied by other signs: - Horners syndrome; - dysarthria; - incoordination; - diplopia; - numbness of the face. - Inferior cerebellar infarction can present with only vertigo, nystagmus, and postural instability. - Multiple sclerosis can produce an evolving vestibular syndrome with a plaque around the 8th nerve root entry zone but other signs present. Clinical features Clinical presentation is with acute onset vertigo, nausea and vomiting. Spontaneous nystagmus - Peripheral origin is indicated by the following characteristics: - Horizontal with a torsional component. - Does not change direction with a change in gaze. - Bidirectional nystagmus excludes the diagnosis. - Slow phase towards affected ear; fast phase towards unaffected ear. - Visual fixation d the nystagmus and removing fixation i it. At bedside, if Fresnel lenses not available, use an ophthalmoscope focused on the optic disc or the retinal blood vessels with the other eye covered. The nystagmus should be evident in the primary position. Note that the direction of the nystagmus is inverted when viewed through the ophthalmoscope - Central nystagmus is often purely horizontal or vertical and changes in direction with changes in the position of the gaze (i.e. bi- or multidirectional). - Purely vertical and purely torsional nystagmus is usually also of central origin.

95. ACUTE VERTIGO1 59 - Horizontaltortional nystagmus may occur in both peripheral and central disorders. - Visual fixation has little effect on nystagmus of central origin. The head impulse test a bedside test of the horizontal vestibulo-ocular reflex (See Fig 3.1). - Indicates absent lateral semicircular canal function on affected side. - If a catch up saccade occurs in one direction and not the other this indicates a peripheral vestibular lesion on that side within the labyrinth or the 8th nerve including the root entry zone in the brainstem. c d e f a b Fig. 3.1 The head impulse test. The examiner turns the patients head as rapidly as possible about 15 to one side and observes the ability of the patient to keep fixating on a distant target. The patient illustrated has a right peripheral lesion with a severe loss of right lateral semicircular canal function. While the examiner turns the patients head towards the normal left side (top row), the patient is able to keep fixating on target. By contrast, when the examiner turns the patients head to the right, the vestibulo-ocular reflex fails and the patient cannot keep fixating on target (e) so that she needs to make a voluntary rapid eye movement, i.e. a saccade, back to target (f) after the head impulse has finished; this can be easily observed by the examiner. It is essential that the head is turned as rapidly as possible; otherwise smooth pursuit eye movements will compensate for the head turn. Reproduced with permission from Halmagyi, G.M. (2005). Diagnosis and management of vertigo. Clin. Med. 5(2), 15965. Royal College of Physicians.

96. CHAPTER 3 Common clinical presentations60 Fukuda or Unterbergers test Marching on the spot with eyes closed and arms out. Positive testpatient veers to side of the lesion. Cerebel- lar lesion patients unable to stand unaided to do test. Does not discrimi- nate between central and peripheral causes. Other signs - Patients with a peripheral lesion can typically stand but veer/tilt to the side of the lesion. Those with a central lesion are often unable to stand without support. - If signs not typically peripheral assume central and investigate. Recurrent attacks of acute vertigo May be due to one of the following. - Mnires disease. - Migraine. - Posterior circulation TIAs (rare); brief crescendo of attacks heralding stroke. Some may be associated with diplopia, dysarthria, or facial numbness. - Episodic ataxia. Differential diagnosis See Table 3.2. Management - If peripheral, treat is with vestibular sedatives, e.g. betahistine 816 mg TDS. Symptoms always resolve in a few days due to vestibular compensatory mechanisms. - If central, consider CT to exclude a cerebellar infarction/haemorrhage. MRI more sensitive at detection of posterior fossa infarcts. Some develop cerebral oedema resulting in hydrocephalus and need urgent shunting and/or decompression. - Significant number of posterior circulation infarcts due to cardiogenic embolism. - ECG, 24-hour ECG, transthoracic and or trans-oesophageal echo. - ? Anticoagulation.

97. ACUTE VERTIGO1 61 Table 3.2 Differential diagnoses of acute vertigo Cause History Examination Investigation Acute vestibular neuritis Develops over hours and resolves in days; viral infection Spontaneous peripheral nystagmus, positive head impulse test Unilateral caloric hypoexcitability, audiogram normal. MRI normal Labyrinthine infarction Abrupt onset; previous vascular disease As for vestibular neuritis As for neuritis; MRI-silent infarcts Perilymph fistula Abrupt onset; associated head trauma, barotrauma, coughing or sneezing; May be associated with chronic otitis and cholesteatoma As for neuritis; possible perforation of tympanic membrane. Positive fistula test (vertigo and nystagmus induced by pressure in the external canal) As for labyrinthitis; CT temporal bone may show erosion from cholesteatoma Brainstem and cerebellar infarction Abrupt onset; history of vascular disease; other neurological symptoms Spontaneous central nystagmus; head impulse test positive only if root entry zone involved; focal neurological signs Unilateral caloric hypoexcitability if anterior inferior cerebellar artery involved. MRI shows infarction in medulla, pons, or cerebellum. Note: Mnires syndrome can initially present with acute vertigo but it rarely lasts more than 4 or 5 hours (other symptoms: low frequency tinnitus, hearing loss, and a sense of fullness in the ears).

98. CHAPTER 3 Common clinical presentations62 Acute headache (thunderclap headache) - 2% of visits to A & E department are due to headache. - In patients with worst ever headache and a normal neurological examination, 12% may have a subarachnoid haemorrhage (SAH). If neurological exam is abnormal this becomes 25%. The diagnosis of SAH is missed initially in up to 32%. Thunderclap headache may be defined as an abrupt onset, often a worst ever headache that is maximal in seconds but may develop in minutes. Differential diagnoses Vascular causes - SAH. - Carotid and vertebral artery dissection. - Cerebral venous thrombosis. - Arterial hypertension. Non-vascular causes - Meningoencephalitis. - Intermittent hydrocephalus (colloid cyst of the 3rd ventricle). - Spontaneous intracranial hypotension. Primary headache syndromes - Coital cephalgia (headache associated with sexual activity). - Crash migraine. - Benign cough and exertional headahe. - Icepick or idiopathic stabbing headache. - Exploding head syndrome. Clinical features The red flags in a patient with such a presentation include: - worst ever headache; - onset with exertion (20% of SAH occur with exertion, e.g. sexual intercourse); - impaired alertness or conscious level, neck stiffness, progressive neurological deterioration; - abnormal neurological examination (third or sixth nerve palsy, papilloedema, subhyaloid haemorrhage, hemiparesis, or diplegia (anterior communicating aneurysm). A first episode of headache cannot be classified as tension type headache (IHS criteria for diagnosis requires at least 9 similar episodes) or migraine (4 previous episodes required for diagnosis) without aura. Investigations All patients should have a CT scan and, if that is negative, a lumbar puncture. CT scans become less sensitive at the detection of blood with time: - day 1, 95%. - day 3, 74%. - day 7, 50%.

99. ACUTE HEADACHE (THUNDERCLAP HEADACHE)1 63 - day 14, 30%. - day 21, almost 0%. Therefore, 5% of scans in patients with SAH normal initially. Technical factor is thin cuts ( 100 000 RBC + 12 WBC per 1000 RBC. If there are a lot more white cells consider meningitis complicated by a traumatic tap. Alternatively, after a few days following a SAH, a meningitic reaction may occur. In SAH protein is usually elevated. - Xanthochromia (resulting from breakdown of haemoglobin to oxyhaemoglobin (pink) and bilirubin (yellow) may take at least 12 hours to develop; hence the recommendations to delay LP until 12 hours after ictus unless meningitis is a strong possibility. This may disappear after 14 days. - Although spectrophotometry is more sensitive than visual inspection in looking for xanthochromia, it is not widely available. - Other causes of xanthochromia: jaundice, elevated CSF protein (>1.5 g/l), malignant melanoma, and rifampicin. If CT positive or there is persistently bloody CSF or xanthochromia by visual inspection, cerebral angiography and a neurosurgical opinion are necessary. SAH versus traumatic tap - OP elevated in SAH. - Use 3 tube test against a white background for xanthochromia. - WBCin SAH, 1 per 1000 RBC. After 35 days, polymorphs and lymphocytes.

100. CHAPTER 3 Common clin

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posterior cutaneous

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genitofemoral nerve